Cervical spinal cord compression in hereditary multiple exostoses. Report of a case and a review of the literature

Spinal cord compression is an extremely serious complication of hereditary multiple exostoses (HME). A case of HME with compression of the cervical spinal cord is reported. Complete recovery following surgery was achieved. A review of the relevant literature revealed 51 previous cases of HME with cord/cauda equina compression. Most patients were under 30 years of age with more men affected than women. The family history was positive in 60%. The cervical and thoracic areas were predominantly affected, with the symptoms usually developing slowly. Recovery following surgery is to be expected in the majority of cases. In patients with HME and suffering from neurological symptoms, the possibility of spinal cord compression should be considered. Prompt diagnosis and surgical excision provide the best prognosis.     

Lumbar spine stenosis: a common cause of back and leg pain

Lumbar spine stenosis most commonly affects the middle-aged and elderly population. Entrapment of the cauda equina roots by hypertrophy of the osseous and soft tissue structures surrounding the lumbar spinal canal is often associated with incapacitating pain in the back and lower extremities, difficulty ambulating, leg paresthesias and weakness and, in severe cases, bowel or bladder disturbances. The characteristic syndrome associated with lumbar stenosis is termed neurogenic intermittent claudication. This condition must be differentiated from true claudication, which is caused by atherosclerosis of the pelvofemoral vessels. Although many conditions may be associated with lumbar canal stenosis, most cases are idiopathic. Imaging of the lumbar spine performed with computed tomography or magnetic resonance imaging often demonstrates narrowing of the lumbar canal with compression of the cauda equina nerve roots by thickened posterior vertebral elements, facet joints, marginal osteophytes or soft tissue structures such as the ligamentum flavum or herniated discs. Treatment for symptomatic lumbar stenosis is usually surgical decompression. Medical treatment alternatives, such as bed rest, pain management and physical therapy, should be reserved for use in debilitated patients or patients whose surgical risk is prohibitive as a result of concomitant medical conditions.     

Cauda equina syndrome. Implications for the orthopaedic nurse in a clinical setting

Cauda equina syndrome (CES) is a presentation of signs and symptoms that indicate nerve compression involving one or more of the nerve roots in the lumbar spine. The Clinical Nurse Specialist working in a general orthopaedic practice must become familiar with the presentation of CES to appropriately assess the patient and alert the physician. Prompt and accurate care of the patient is critical when cauda equina syndrome is suspected to minimize permanent nerve damage. This article describes the syndrome, its signs and symptoms, basic triage techniques, plan of care, and recovery for the patient with cauda equina syndrome.     

Spinal cord compression in metastatic cervical cancer

Spinal cord compression secondary to metastatic cervical cancer may not be considered as a possible cause of neurologic symptoms by primary care physicians who do not often treat these patients. Delays in diagnosis and treatment may result in irreversible but potentially preventable neurologic changes. This report describes 5 cases of spinal cord compression in patients with metastatic cervical carcinoma, 2 of whom were previously undiagnosed with cervical cancer. These 2 patients represent 1.6% (2 of 121) of all new cervical cancer cases diagnosed during this time period. Two of 5 patients (40%) with spinal cord compression showed improvement following therapy by regaining the ability to walk, while none of the remaining patients had further acute deterioration of neurologic function. The mean survival of patients presenting with spinal cord compression from cervical cancer in this series was 4 months (maximum 6 months). This series illustrates the relative frequency with which spinal cord compression is seen in patients with a new diagnosis of invasive cancer. This diagnosis should be considered when evaluating neurologic complaints in known cervical cancer patients or any woman with apparent pelvic pathology. Rapid diagnosis and treatment of these lesions, while not likely to improve overall survival significantly, can improve function and alleviate symptoms.     

Lymphoma of the spinal extradural space

Ninety-four patients with lymphoma involving the extradural space with spinal cord compression proven at the time of laminectomy were reviewed. There were about three times as many patients with non-Hodgkin's lymphoma than with Hodgkin's disease. The majority of those with Hodgkin's disease had a proven histologic diagnosis before the onset of the spinal cord compression syndrome, whereas only 15% of those with non-Hodgkin's lymphoma had previously been so diagnosed. Plain roentgenograms of the spine were suggestive of tumor involvement in less than one-third of the patients, whereas myelograms were invariably abnormal. As noted by others, the outlook for functional recovery and extended life expectancy is relatively good for patients with this type of cancer, in contrast to reports in the literature regarding prognosis for patients who have metastatic carcinoma with extradural spinal cord compression.     

Harrington instrumentation in fractures and dislocations of the thoracic and lumbar spine

Harrington instrumentation was used to treat 23 injuries of the thoracic and lumbar spine accompanied by neural damage encountered during an 8 1/2-year period. The indication for the use of Harrington instrumentation is instability of the thoracic or lumbar spine due to trauma, laminectomy, or both. Of the 13 patients with incomplete neural lesions in the series, 12 improved. One of these was a cauda equina lesion. No patient in the series was neurologically worse after operation, in contrast to other reported series in which laminectomy alone was done. It is concluded that Harrington instrumentation is the strongest available system of internal fixation, and the only one that permits reduction of nearly all traumatic spinal deformities at the time of operation.     

Clinical features of intradural neurinomas in the cauda equina and around the conus medullaris

In this contribution the clinical features of eleven patients suffering from a neurinoma in the cauda equina and around the conus medullaris are analysed. Because of the relative mobility of the roots and the wide space in the spinal canal, tumours arising in the cauda equina or around the conus medullaris can become larger than any other spinal tumours. Lumbago was the predominant symptom as the initial complaint. Nocturnal pain relieved by walking was noticed in one patient. Two cases showed spontaneous remission and relapse. Multiple tumours were found in 6 cases (55%). Macroscopic cyst formation was found in 5 cases (45%). Among the eleven patients, total removal of the tumour, including the involved root, was performed in ten. The numbers of the resected nerve roots were one root in 6 cases, two roots in 2, and three roots in 2. Only one patient showed postoperative slight weakness of the leg.     

Electrophysiological technique for evaluating lesions of the conus medullaris and cauda equina

The authors describe the use of evoked electromyographic responses recorded in the anal sphincter induced by stimulation of the bladder wall and urethra in evaluating lesions of the conus medullaris and cauda equina in 110 patients. This reflex response took effect by way of the pelvic nerves and cauda equina to the sacral cord where the pudendal nerve nucleus was activated, resulting in a contraction of the external anal sphincter. Various lesions along this pathway have been shown to produce either increased latencies and depressed responses or complete loss of response depending on the extent of the lesion. The correlation of results of this technique with clinical, myelographic, and operative findings indicate it to be a useful clinical tool.     

Cervical radiculopathy as a form of presentation of non-Hodgkin's lymphoma

INTRODUCTION: Neurological involvement in no-Hodgkin lymphoma is usually a serious problem leading to subacute compression of the spinal cord secondary to invasion of the spinal extradural space. This condition may be the presenting form of the illness, although relatively infrequently. Onset with involvement of the cauda equina or as an isolated radiculopathy is even rarer and has mainly been described at lumbar level. CLINICAL CASE: We present the case of a 27 year old man with no significant previous clinical history who had progressive, insidious onset of moderate-serious senso-motor radiculopathy at C8. On MR there was an infiltrating mass affecting the nerve root and reaching the spinal extradural space. Following decompression laminectomy the patient's leg recovered and on histology there was a large cell B lymphoma. The other complementary tests showed disseminated disease, although analysis of the cerebro-spinal fluid (including cytology) was normal. HIV seriology was also normal. CONCLUSION: Although isolated nerve root involvement is unusual in the presentation of lymphoma it should be considered to be part of the differential diagnosis of nerve root syndromes in young patients with no history of trauma or degenerative lesions.     

Experimental evaluation of the urethral response to section of the spinal cord or cauda equina and to the electrostimulation of the bladder in non-human primates

Studies of urethral function in 32 female monkeys indicate a dual innervation of the mid-urethra from sympathetic and somatic supply. The resting urethral pressure profiles were preserved after low level section of the spinal cord or division of the cauda equina. They had lowered amplitude after overstretch or electrostimulation of the autonomous bladder.     

Intradural parenchymal involvement in the spinal subarachnoid space associated with primary lung cancer

BACKGROUND: Intradural parenchymal involvement (IPI) in the spinal subarachnoid space associated with primary lung cancer is rare. A retrospective study was undertaken to investigate the clinical and pathologic features of IPI. METHOD: A total of 1215 cases of primary lung cancer were studied at autopsy; the results were reviewed retrospectively. RESULTS: Twenty (1.65%) of the cases revealed IPI in the spinal subarachnoid space. The histologic diagnoses were small cell carcinoma in ten cases, adenocarcinoma in eight cases, and squamous cell carcinoma in two cases. In 14 (70%) cases, the IPI was located between the lumbar and cauda equina of the spinal cord. However, no metastases were observed in the cervical spinal cord. Brain metastasis, vertebral metastasis, and meningeal carcinomatosis were seen in 70%, 60%, and 40% of the 20 cases, respectively, suggesting that these metastases may be related to the metastatic pathway to the spinal cord. Most patients had neurologic symptoms or signs referable to IPI; IPI could be diagnosed before death in only one patient by magnetic resonance imaging. The median interval between diagnosis of lung cancer and development of IPI and median survival after the onset of neurologic symptoms referable to IPI were 415 days and 110 days, respectively. CONCLUSION: The authors retrospectively received 1215 autopsies of patients with primary lung cancer and found 20 (1.65%) with IPI.     

Histologic analysis of the lumbosacral nerve roots after compression in young and aged rabbits

STUDY DESIGN: The histologic changes in the lumbosacral nerve roots of aged rabbits because of chronic (graded) and acute compression were compared with those seen in young rabbits. OBJECTIVES: To study differences in the process of recovery from nerve compression between the aged and the young. SUMMARY OF BACKGROUND DATA: Clinical findings often differ between nerve compressive lesions in young and aged patients. Little has been reported on the pathologic basis of this difference. METHODS: Forty-five Japanese white rabbits were used. The cauda equina and spinal nerve root were compressed with a device specifically designed for this purpose. Nerve compression was applied to the dura mater and nerve roots after partial laminectomy. The specimens were sampled at 1 month or 3 months after acute or graded nerve compression. RESULTS: An increase in small myelinated fibers, consistent with the process of regeneration, was observed by light microscopy; this difference was greater in the young group than in the aged group. Reactive degenerative changes, as seen by electron microscopy, were more often observed in the aged group than in the young group. CONCLUSIONS: These findings demonstrate the suppression of regeneration and the latent fragility of the aged neural tissue. This may explain the clinical findings observed in aged patients with degenerative lumbar lesions.     

Clinical analysis of two-level compression of the cauda equina and the nerve roots in lumbar spinal canal stenosis

STUDY DESIGN: This study is a prospective, clinical study assessing the efficacy of selective decompression of the responsible level in two-level stenosis in accordance with neurologic findings defined by the gait load test, and functional diagnosis based on selective nerve root block. OBJECTIVE: To clarify the clinical features of two-level stenosis regarding the neurologic level responsible for the symptoms, neurogenic intermittent claudication, and the outcome of selective decompression. SUMMARY OF BACKGROUND DATA: Experimental studies have indicated that double-level compression of the cauda equina induces a more severe impairment of nerve function than does single-level compression. However, few studies have focused on the clinical importance of two-level stenosis. The clinical effects of two-level stenosis on the cauda equina and nerve roots are unknown. METHODS: A total of 81 patients with lumbar spinal canal stenosis due to spondylosis and degenerative spondylolisthesis were divided into two groups, two-level stenosis at L3-L4 and L4-L5, and one-level stenosis at L4-L5, based on myelography. The types of neurogenic intermittent claudication, the level responsible for neurologic findings, and the postsurgical outcome were compared between both groups. The level responsible for the symptoms in two-level stenosis was determined in accordance with neurologic findings on the gait load test and functional diagnosis based on a selective nerve root block. All patients underwent a prospective, selective decompression at the neurologically responsible level only. The average follow-up period was 4.6 years (range, 1-8 years). RESULTS: The patients with two-level stenosis more frequently had cauda equina symptoms than those with one-level stenosis, except patients with degenerative spondylolisthesis. It was therefore assumed that two-level stenosis was associated with cauda equina impairment, Changes in neurologic condition before and after the gait test were observed in four patients with two-level stenosis. Finally, for 28 patients with two-level stenosis, the levels responsible for the neurologic symptoms were the caudal level (L4-L5) in 22 patients, the cranial level (L3-L4) in 1 patient, and both cranial and caudal levels (L3-L4 and L4-L5) in 5 patients. All stenotic levels on the myelogram were not always symptomatic in two-level stenosis. However, in one-level stenosis, all of the responsible levels completely corresponded to the myelogram. Selective decompression only at the neurologically responsible level improved neurogenic intermittent claudication in all patients. The asymptomatic levels at which the stenotic condition was left unchanged at surgery did not become symptomatic at follow-up; in addition, there was no significant difference in the postoperative outcome between two-level stenosis and one-level stenosis. CONCLUSIONS: Two-level stenosis in patients with lumbar spondylosis is associated with production of cauda equina lesions. The gait load test provides information regarding changes in symptoms and neurologic condition during exercise. The responsible levels should be determined based on neurologic findings after the gait load test and a selective nerve root block. It is uncommon for both stenotic levels to be symptomatic in patients with two-level stenosis. Less invasive surgery such as selective decompression for the responsible level in patients with two-level stenosis is a useful technique with a good potential for long-term success.     

Computed tomography of the spine as an important diagnostic tool in the management of war missile spinal trauma

Twenty-two patients with spinal injury were evaluated by plain radiography immediately after hospital admission. In 14 patients whose condition was stable, we performed computed tomography (CT) scanning through the involved segments. To provide better planning before neurosurgical management, we divided the vertebral column in thirds. According to this division, we concluded that these injuries are mostly extensive, severely damaging all three thirds of the vertebral column and accompanying neural structures in the majority of cases. The information acquired by Ct concerning bony fragments, bone destruction, dural tear, spinal cord and nerve root compression, and neural damage directly influenced the surgical management. All patients except one underwent surgery while associated injuries of other organs were given priority in management. Injuries of the thoracic and the lumbar spine were the most common ones, frequently found in association with lesions of nearby organs. Penetrating injuries with a dural lesion were present in the majority of cases, while spinal cord injury was obvious in some. They were all well visualized using spinal CT scanning. Our view is that the role of CT is essential in guiding surgical management of war missile injuries to the spine.     

Analysis of failures and poor results of lumbar spine surgery

The failures and poor results of lumbar spine surgery are analyzed in a retrospectively study of 105 consecutive patients referred to the authors for evaluation during 1976. Those who had a history of industrial or vehicular accident outnumbered others by about two to one. Review of histories, physical findings, and myelograms in most of the patients failed to substantiate the diagnosis of ruptured disc or nerve root compression. Many of the failures occurred in thos patients in whom little if any evidence of nerve root compression was found. The indications for surgery were poor in this group. Other failures occurred in patients who had improper, incomplete, or inadequate operations, especially those with lumbar spondylosis, a retained fragment of disc, or surgery at the wrong level. In addition to failure, poor results were recorded in patients who had significant nerve root or cauda equina injury from surgery, associated "arachnoiditis" which is thought to result from surgical trauma in many instances, or multiple operations leading to a hopelessly disabled state.     

Inflammatory myofibroblastic tumour of the spinal cord: case report and review of the literature

Inflammatory myofibroblastic tumours (IMT), also called inflammatory pseudotumours, nodular lymphoid hyperplasia, plasma-cell granuloma and fibrous xanthoma, are rare soft-tissue lesions characterised by inflammatory cells and a fibrous stroma. Clinically and radiologically, they may look like malignant tumours. They rarely affect the central nervous system and are very rare in the spinal cord. We report an IMT of the spinal cord in a 22-year-old woman presenting with spinal cord compression and a cauda equina syndrome. MRI showed a lesion at T9 with extramedullary and intramedullary components giving low signal on T2-weighted images and enhancing homogeneously. Pial lesions on the lumbar enlargement and thoracic spinal were present 11 months after surgery, when the lesion recurred. We present the radiological, operative and pathological findings and review the literature.     

Chronic double-level cauda equina compression. An experimental study on the dog cauda equina with analyses of nerve conduction velocity

STUDY DESIGN: Nerve conduction velocity was studied in the dog cauda equina subjected to chronic double-level compression. OBJECTIVES: To analyze the effects of chronic double-level cauda equina compression. SUMMARY OF BACKGROUND DATA: Double-level cauda equina compression produces more symptoms in patients and more changes in acute experimental set-ups than does single-level compression. However, there have been no controlled, experimental studies on chronic double-level compression. METHODS: A total of 20 dogs were anesthetized. Two balloons were placed under the lamina of the seventh lumbar vertebra and the first sacral vertebra, respectively. One week (10 mm Hg, n = 5; 0 mm Hg, n = 5) and 1 month (10 mm Hg, n = 5; 0 mm Hg, n = 5) after inflation with a viscous substance, nerve conduction velocity was studied by local electrical stimulation and recording of muscle action potentials in the tail muscles. RESULTS: Nerve conduction velocity was determined over the cranial balloon, the caudal balloon, and both balloons. The data were similar for all three recordings. After 1 week there was a significant reduction in nerve conduction velocity induced by 10 mm Hg, compared with that induced by 0 mm Hg, which showed normal conditions. However, after 1 month this initial reduction in nerve conduction velocity had recovered partially. The reduction was similar to that described for single-level compression in a previous study in which the same compression model was used. CONCLUSIONS: Unlike the acute situation, chronic double-level compression does not induce more changes than single-level compression after 1 week, although the recovery after 1 month of compression is less complete after double-level compression. This less complete recovery may be a result of an adaptation of the nerve tissue and the vascularization of the cauda equina nerve roots to the applied pressure.     

Sixth congress of the European Genetic Therapy Group

PURPOSE: Course and pathophysiology of a typical syndrome after irradiation of the cauda equina were studied. PATIENTS AND MATERIALS: 7 patients with irradiation damage of the cauda equina were examined clinically and neurophysiologically. RESULTS: After a mean delay of 5 years and 6 months all patients developed an ascending lower motor neuron weakness of the legs without pain, in part accompanied with mild sensory and sphincter symptoms. Electromyography, evoked potentials and neurography were important for the differential diagnosis to tumor infiltration. The course was progredient. DISCUSSION: For a long time, the underlaying damage was thought to be in the anterior horn cell body. The course of the studied patients with additional sensory and vegetative symptoms implies for a direct bilateral damage of the cauda equina. A better term for the syndrome is "post-irradiation cauda-equina-syndrome".     

Lower urinary tract symptoms in lumbar root compression syndromes: a prospective survey

STUDY DESIGN: A prospective, observational survey. OBJECTIVES: To describe lower urinary tract symptoms in uncomplicated lumbar root compression syndromes with special reference to prevalence, nature, and severity, and to analyze whether the occurrence of lower urinary tract symptoms correlates with age, pain, analgesic intake, or the type and level of compression. SUMMARY OF BACKGROUND DATA: Lower urinary tract symptoms with lumbar root compression are well known in the classic but rather rare cauda equina syndrome. However, micturition difficulties seem to be far more frequent in lumbar root compression syndromes. METHODS: One hundred eight male patients admitted for surgery for lumbar disc herniation or spinal stenosis were investigated with an extensive questionnaire about their micturition. RESULTS: Fifty-five percent had significant lower urinary tract symptoms. Eighty percent of the patients with spinal stenosis had symptoms. Thirty-three patients had irritative symptoms, 36 had obstructive symptoms, and 23 had retention symptoms. Twenty-four had severe symptoms. Median compression resulted in more symptoms than paramedian compression. There was no correlation between age, level of compression, drug intake, or pain score and lower urinary tract symptoms. CONCLUSIONS: Lower urinary tract symptoms of mixed type occur with a high prevalence in male patients with lumbar root compression syndromes referred for neurosurgical evaluation and treatment.     

The syndrome of painful legs and moving toes--a case report

The syndrome of painful legs and moving toes is an uncommon and distressing condition with pain in the feet or legs and involuntary movements of the toes. It can follow spinal cord or cauda equina trauma, lumbar radiculopathy, injury to the feet, peripheral neuropathy or without any preceding causes. Ephaptic transmission in damaged nerve roots or peripheral nerves with central reorganisation may be the underlying mechanism of the syndrome. Treatment is difficult. We report a case of this syndrome following peripheral neuropathy, with a good early response to the GABA agonists baclofen and clonazepam. The role of different GABA agonists in the treatment of this condition needs to be better defined.